Journal of Optometry (2014) 7, 106---107
www.journalofoptometry.org
SCIENTIFIC LETTER
Bilateral Tonic Pupils Secondary to Ross
Syndrome: A Case Report
Pupilas tnicas bilaterales secundarias al
sndrome de Ross: informe de un caso
Introduction
In 1958, Alexander Ross described a case with anhidrosis
and Adies syndrome. He considered the Adies syndrome
unrelated to the anhidrosis.1 Currently, Ross syndrome is
described as tonic pupils, decreased or a loss of deep
tendon reexes, and segmental anhidrosis.2 Ross syn-
drome is rare, with less than 50 cases existing in the
literature.2
Case Report
A 44 year-old white male presented to the eye clinic with
complaints of difculty with night driving. His medical
history was signicant for the following medications: var-
denal, uoxetine, simvastatin/ezetimibe, tramadol, sh
oil, glucosamine, and zolpidem and he had an allergy to
percocet. In 1993, he had a history of tick bites from
which he developed Rocky Mountain spotted fever (rick-
ettsiosis). After treatment with an antibiotic and recovering
from this illness, he noticed an absence of sweating
on half of his body. The patient had extensive test-
ing, such as VDRL and FTA-ABS, which was negative. He
demonstrated hypersensitivity to dilute pilocarpine and
decreased tendon reexes and was diagnosed with Ross syn-
drome.
His distance visual acuity without correction was 20/25
in the OD and 20/20 in the OS. Pupils were unreac-
tive to light, with a size of 3---4 mm in bright and dark
illumination. Because of the small pupil size, the near
reaction was difcult to elicit. The left pupil showed ver-
miform movements superiorly. The pupils had an irregular
shape. Slit lamp exam revealed a normal anterior seg-
ment. The eld was full by confrontation. Tonometry was
measured as 14 in the OD and 14 in the OS. Dilated fun-
dus exam revealed a normal posterior segment, with a
cup-to-disk ratio of 0.6 in the OD and OS. He had a loss
of sweating on his right side and a loss of deep tendon
reexes.
1888-4296/$ see front matter 2013 Spanish General Council of Optometry. Published by Elsevier Espaa, S.L. All rights reserved.
http://dx.doi.org/10.1016/j.optom.2013.06.005
Discussion
The iris is innervated by the parasympathetic and sympa-
thetic nervous systems. A defect in the parasympathetic
nervous system may result in dilation of the pupil in bright
light, while a defect of the sympathetic nervous system may
result in miosis of the pupil in the dark.3 Light-near dis-
sociation is caused by many pathologies, among which are
Argyll Robertson and tonic pupils. Many diseases may cause
tonic pupils, such as diabetes, herpes, sarcoid, injury, infec-
tion, syphilis, Guillan-Barre syndrome, Shy-Drager, tumor,
Charcot-Marie-Tooth, and Holmes-Adie syndrome.2 Argyll
Robertson pupils are bilateral small pupils with a rapid con-
striction to near, but with slight or absent constriction to
light.4 Argyll Robertson pupils may have an association with
syphilis, so optometrists should order the appropriate lab
tests such as the VDRL and FTA-ABS.
Adies syndrome, or Holmes-Adie syndrome, refers to
cases in which the etiology of the tonic pupils is unknown.
Holmes-Adie syndrome is characterized by poor light
reaction, paralysis of accommodation, hypersensitivity to
cholinergic drugs, pupils that respond to near vision slowly
and decreased tendon reexes.6 Typically, the syndrome
is unilateral and typically occurs in women more than
men.3 Holmes-Adie syndrome tends to progress; the fel-
low pupil becomes involved and the deep tendon reexes
decrease.5 The cause of the progressive miosis remains
unknown.7 Patients with Holmes-Adie syndrome may present
with blurred near vision as the chief complaint.5
Ross syndrome, while benign, is a progressive autonomic
dysfunction, which can occur in patients of any age, ethnic
background, or gender.6 Of the twenty patients with Ross
syndrome described in the literature, slightly more were
male than female.8 The typical age at the time of diagnosis
of Ross syndrome patients was 36 years.8 The tonic pupil was
bilateral in a majority of cases.
Ross syndrome is characterized by tonic pupils, decrease
or loss of deep tendon reexes, and segmental loss of
sweating. Tonic pupils show progressive miosis; this mio-
sis progresses more quickly than the normal miosis of
aging. Damage to the sympathetic ganglion cells or the
postganglionic projections could account for the loss of
sweating.6 The loss of deep tendon reexes may stem from
the damage in the dorsal root ganglia or spinal interneu-
ron loss.6,7 The mechanism of injury is unknown.6,9 The
peripheral autonomic nervous system and dorsal root gan-
glia all develop from neural crest cells.9 One author suggests
SCIENTIFIC LETTER
that some commonality makes these tissues prone to
injury.9
Patients with parasympathetic and sympathetic dys-
function may have systemic symptoms in addition to
the ocular symptoms. Patients with Ross syndrome or
Holmes-Adie syndrome may present with the following:
orthostatic hypotension, headache, psychiatric disorders,
reduced heart rate responses to Valsalva maneuvers,
diarrhea.9 Ross syndrome overlaps with Holmes-Adie syn-
drome and may represent different manifestations of the
same disorder.6,9 However, one author has suggested that
Ross syndrome can be differentiated from Holmes-Adie
syndrome by the loss of sweating, since both condi-
tions present with tonic pupils and decreased tendon
reexes.7
Conclusion
Tonic pupils may indicate complex systemic disease.10
Optometrists should order appropriate lab tests to rule
out systemic disease in cases of tonic pupils, although
Holmes-Adie syndrome is idiopathic. Holmes-Adie syndrome
is characterized by tonic pupils, paralysis of accommoda-
tion, and decreased deep tendon reexes.
Ross syndrome is a rare, but progressive autonomic
disease that causes tonic pupils, loss of deep tendon
reexes, and anhidrosis. Ross syndrome can be differ-
entiated from Holmes-Adie syndrome by the lack of
anhidrosis in Holmes-Adie syndrome. Patients with Ross
syndrome have autonomic dysfunction and may show sys-
temic symptoms such as headache, psychiatric disorders,
and abnormal heart rate responses.9 Patients have dif-
culty regulating body temperature and should avoid very
hot or cold conditions.7 Optometrists need to be aware of
107
Ross syndrome and refer to other practitioners as neces-
sary.
References
1. Ross A. Progressive selective sudomotor denervation; a case
coexisting with Adies syndrome. Neurology. 1958;8:809---817.
2. Nolano M, Provitera V, Perretti A, Stancanelli A, Saltalamac-
chia AM, Donadio V, et al. Ross syndrome: a rare or misknown
disorder of thermoregulation? A skin innervation study on 12
subjects. Brain. 2006;129:2119---2131.
3. Bremner F, Smith SE. Bilateral tonic pupils: Holmes-Adie
syndrome or generalised neuropathy. Br J Ophthalmol.
2007;91:1620---1623.
4. Thompson HS, Kardon R. The Argyll-Robertson pupil. J Neuro-
Ophthalmol. 2006;26:134---138.
5. Thompson HS. Adies syndrome: some new observations. Trans
Am Ophthalmol Soc. 1977;75:587---626.
6. Ballestero-Diaz M, et al., Garcia-Rio I, Dauden E, Corrales-
Arroyo MJ, Garcia-Dietz A. Ross syndrome, an entity included
within the spectrum of partial disautonomic syndromes. J Eur
Acad Dermatol Venereol. 2005;19:729---731.
7. Rosenberg ML. Miotic Adies pupils. J Clin Neuro-Ophthalmol.
1989;9:43---45.
8. Weller M, Wilhelm H, Sommer N, Dichgans J, Wietholter H.
Tonic pupil, areexia, and segmental anhidrosis. J Neurol.
1992;239:231---234.
9. Shin RK, Galletta SL, Ting TY, Armstrong K, Bird SJ. Ross
syndrome plus: beyond Homer, Holmes-Adie, and harlequin.
Neurology. 2000;55:1841---1846.
10. Hedges T, Gerner E. Ross syndrome (tonic pupil plus). Br J
Ophthalmol. 1975;59:387---391.
Heidi Mayer
Erie VA Medical Center Erie, PA, United States
E-mail address: mayerhm@yahoo.com